How is it diagnosed and managed?
The Australian guidelines for the prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd Edition) are the main source of information on the diagnosis and management of both conditions.
Diagnosis of Acute Rheumatic Fever
There is currently no single test to diagnose acute rheumatic fever (ARF). Diagnosis is a doctor’s decision based on clinical assessment and the identification of a number of signs and symptoms that are associated with the illness.
Potential signs and symptoms of ARF are divided into two categories: major and minor. Major signs and symptoms are strongly associated with ARF and include carditis (swelling of the heart), arthritis (pain, redness and swelling of one or more joints), Sydenham’s chorea (strange movements of the body and face), erythema marginatum (painless skin pigmentation), and subcutaneous nodules (small lumps under the skin). Minor signs and symptoms are used to help support the diagnosis. These include fever, arthralgia (generalised joint aches), blood tests that suggest general illness, and changes seen on heart electrocardiogram.
A combination of these signs and symptoms, plus a positive test for recent group A streptococcus infection is required to confirm ARF diagnosis.
An accurate diagnosis is important because:
- Mistakenly confirming a diagnosis in a person who does not have ARF will result in years of unnecessary treatment
- Missing a diagnosis in a person who does have ARF may result in development or worsening of heart valve damage and the need for future heart surgery and/or premature death.
Early and accurate diagnosis of acute rheumatic fever is vital to prevent disease progression.
Management of Acute Rheumatic Fever
People who are suspected to have acute rheumatic fever (ARF) should be immediately referred to a medical specialist who is an expert in ARF diagnosis. If people live in remote areas, the treating doctor should contact a medical specialist as soon as practical to discuss the possibility of ARF.
In Australia, it is recommended that all people suspected of having ARF are admitted to hospital as soon as possible after they develop symptoms, so that specialists can do a complete assessment to confirm or rule out ARF.
Management of ARF is based on treating infection and relieving symptoms. This includes antibiotics to treat the streptococcal infection, medication to reduce arthritis and fever if required, rest, and a healthy diet.
Following ARF diagnosis, the following longer-term management should be established:
- Register the person with the RHD control program (where available) or refer back to their local health care facility
- Regular secondary prevention treatment to prevent recurrent ARF
- Ongoing disease education and self-management support
- Ongoing care including regular medical review, heart monitoring and dental care.
Diagnosis and management of Rheumatic Heart Disease
Rheumatic heart disease (RHD) is most accurately diagnosed using ultrasound. Ultrasound of the heart is referred to as echocardiography. Standard guidelines have been developed for echocardiographic diagnosis of RHD, based on which heart valves are involved and the type and severity of damage to each valve.
One or more valves can be affected; however symptoms of RHD may not be noticed for many years. People with mild RHD may not have symptoms, but may have already developed a murmur (abnormal heart beat sound) which can be heard through a stethoscope. Symptoms of moderate to severe RHD can include chest pain, breathlessness with physical activity or when lying down, weakness and tiredness, and swelling of the legs and face. People suspected of having RHD need to be assessed by a medical specialist who is an expert in RHD diagnosis.
Specific management requires treating the symptoms and preventing worsening of the disease, which also depends on the heart valves involved and the extent of the damage. The management principles for RHD include:
- Regular secondary prevention treatment for people at risk of recurrent ARF
- Reliable access to a medical specialist
- Reliable access to dental services
- Reliable access to echocardiography services
- Adequate monitoring of anticoagulation therapy (for people with atrial fibrillation and/or artificial heart valves)
- Access to investigation and surgery services (for people with moderate or severe disease).